Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Clinical Biomedicine > W7: Bleeding Disorders > Flashcards

W7: Bleeding Disorders Flashcards

1
Q

diff btwn primary + secondary haemostatic disorder

A

primary hemostasis = formation of primary platelet plug
secondary hemostasis = coagulation cascade/formation of blood clot

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

examples of PHD + SHD

A

PHD = thrombocytopenia
VWD

SHD = haemophilia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

PHD vs SHD: bleeding

A

p - immediate
s - delayed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

PHD vs SHD: petechiae

A

p - yes
s - no

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

PHD vs SHD: epistaxis

A

p - common
s - uncommon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

PHD vs SHD: menorrhagia

A

p - common
s - uncommon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

PHD vs SHD: Haemarthroses

A

p - no
s - yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

PHD vs SHD: intramuscular haematomas

A

p - uncommon
s - common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

PHD vs SHD: gender

A

p - equal
s - >80% male

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Bleeding defects may result from

A

leaky vessels
low platelet number (thrombocytopenia)
low platelet function
low coagulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Thrombocytopenia - Causes

A

Failure of marrow production
Drug-induced
DIC
Sequestration - pooling of platelets in Splenomegaly
Massive blood transfusion-> dilution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

define ITP - Idiopathic thrombocytopenic purpura (immune destruction)

A

Sudden onset of immunological destruction of platelets and thus thrombocytopenia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ITP acute vs chronic

A

A
Sudden onset in children, often after illness.
90% spontaneous remission after few weeks

C
Gradual onset in adults (3F:1M) (no previous viral infection)
Autoantibodies (antibodies against self)
Platelets destroyed in spleen + liver (within a few hours).
usually lifelong condition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

ITP diagnosis (A +C)

A

Low platelet no.
Large platelets
[Hb] + WBC typically normal
Tests for autoantibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

ITP treatment (A vs C)

A

A - observation
C - Corticosteroids (supress immune sys)
Splenectomy
Immunosuppressive drugs
Platelet transfusions (short term benefit)
Stem cell transplantation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

define TTP - Thrombotic thrombocytopenic purpura

A

Formation of vWF multimers -> platelet aggregation (thrombosis) -> thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

2 types of TTP + diff

A

acquired - Ab blocks protease that breaks down vWF multimers

inherited - protease absent/ defective

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

TTP diagnosis

A

thrombocytopenia
schistocytosis, (fragmented RBCs)
incr serum LDH
Coagulation NORMAL unlike in DIC.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

TTP treatment

A

Plasma exchange
to remove vWF multimers + Ab + provide protease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what’s DIC
disseminated intravascular coagulation

A

Thrombosis + bleeding at the same time (in diff parts of body)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

DIC usually happens when what other conditions also occurring

A

Infections - sepsis
Malignancy
Wide spread tissue damage (surgery / trauma / burns)
Anaphylaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

DIC treatment depends on whether patient bleeding (treatment x) or thrombotic (treatment y)

A

fresh frozen plasma
heparin

23
Q

steps of platelet plug formation

A

adhesion
activation
aggregation

24
Q

disorders of platelet function
inherited vs acquired

A

Inherited
Platelet adhesion defects
Activation defects
Secretion defects
Aggregation defects

Acquired
Aspirin and other drugs
Chronic renal failure
Cardiopulmonary bypass
Haematological diseases

25
Q

what can vWF in plasma protein bind to on surface of platelets (2) + effect

A

collagen
GPIb complex

initial interaction not v strong so only slows platelet down

26
Q

what’s most common inherited bleeding disorder

A

VWD

27
Q

3 types of VWD + what does it depend on

A

Type 1: Quantitative partial deficiency
Type 2: Functional abnormality
Type 3: Complete deficiency

depends on autosomal recessive or dominant mutation

28
Q

where is vWF prod (2) + roles (2)

A

endothelial cells + megakaryocytes

roles
platelet adhesion
carries FVIII prolonging its half life

29
Q

vWF gene is on chromosome

A

12

30
Q

VWD diagnosis

A

Usually normal platelet number + morphology

bleeding prolonged

aPTT prolonged
PT normal, TT normal

vWF levels often low
FVIII levels often low

31
Q

VWD treatment

A

no treatment req

Education - drug avoidance (aspirin), contact sport avoidance

Genetic counselling

Desmopressin - induces release of vWF from endothelium

Tranexamic acid (antifibrinolytic)

32
Q

what’s Bernard-Soulier Syndrome

A

inherited: platelet adhesion defect
GPIb complex

33
Q

2 examples of inherited: activation defects

A

GPVI deficiency
cyclooxygenase deficiency

34
Q

examples of inherited: secretion defects

A

Grey platelet syndrome
Platelets lack alpha granules ( so look grey)

Wiskott-Aldrich Syndrome
platelets (+ other cells) lack dense granules (+ are smaller) + thrombocytopenia

35
Q

Example of inherited: Aggregation Defects

A

Glanzmann’s thrombasthenia

integrin aIIbB3 defect

Inability of platelets to bind fibrinogen + form platelet plug

36
Q

Glanzmann’s thrombasthenia symptoms

A

Easy bruising
menorrhagia

37
Q

Glanzmann’s thrombasthenia diagnosis

A

normal platelet no. + morphology

prolonged bleeding

38
Q

Glanzmann’s thrombasthenia treatment

A

Education
Local haemostatic agents (“quick clotters”)
Platelet transfusions
Genetic counselling

39
Q

Acquired Platelet Disorders: Aspirin + other drugs

A

Anti-platelet drug

Inhibits cyclooxygenase-1
decr in prod of thromboxane A2 (in platelets)
decr prod of PGI2 (in endothelial cells)

40
Q

Acquired Platelet Disorders: Aspirin + other drugs treatment

A

platelet transfusion
desmopressin (induces release of vWF from endothelial cells)

41
Q

Acquired Platelet Disorders: Chronic Renal Failure

Renal failure causes incr bleeding time due to either

A

Uraemia (high nitrogen in blood leading to multiple platelet defects)
Anaemia ( decr Epo synthesis by kidneys)
Affects platelet – vessel wall interactions

42
Q

Acquired Platelet Disorders: Chronic Renal Failure treatment

A

RBC packs (bulk blood, pushing platelets to the vessel walls)
Platelet transfusions (limited effect as then in uraemic plasma)

43
Q

Acquired Platelet Disorders: Cardiopulmonary bypass

A

Platelet activation and degranulation occurs in the extracorporeal circuit (out of the body).

44
Q

Acquired Platelet Disorders: Cardiopulmonary bypass treatment

A

Platelet transfusion

45
Q

Haematologial diseases:
Several blood diseases impair platelet function

A

Most of the leukaemias (bone marrow activity affected).
Myeloma (plasma B-cells ie bone marrow affected).

46
Q

Reduced coagulation tends to arise from

A

A deficiency in a coagulation factor
Inherited incl. fibrinogen
Acquired

47
Q

Inherited: Coagulation factor deficiencies
Haemophilia A

A

Deficiency in FVIII.
X-linked recessive disorder.
Mostly inherited, though 30% due to sporadic mutations.
Severity varies from mild to severe.

48
Q

Inherited: Coagulation factor deficiencies
VWD

A

vWF carries FVIII
Most common inherited bleeding disorder
1:100 (asympt)
1:10 000 (sympt).
Autosomal dominant/recessive.

49
Q

Inherited: Coagulation factor deficiencies
Haemophilia B

A

Deficiency in FIX.
X-linked recessive disorder.
Severity variable

50
Q

other factor deficiencies - autosomal recessive

A

FXI
FVII
FV
FXIII

51
Q

Abnormalities of fibrinogen

A

Quantitative deficiencies
Apofibrinogenaemia - Autosomal recessive. Severe symptoms.
Hypofibrinogenaemia - Less defined. Milder symptoms.

Qualitative deficiencies
Dysfibrinogenaemia- Autosomal dominant. haemorrhagic effect (50%)
thrombotic effect (10%)

52
Q

Acquired: Coagulation factor deficiencies (3)

A

Liver Disease
site of synthesis of several clotting factors (+ thrombopoietin)
Liver function tests include: Albumin, Bilirubin, Prothrombin Time

Vitamin K deficiency
The reduced form of Vitamin K is needed for post-translational modification of several clotting factors (when synthesised).

Warfarin use
Warfarin inhibits the enzyme that cycles Vitamin K (vitamin K epoxide reductase

53
Q

Acquired: Coagulation factor deficiencies
Acquired haemophilia
diagnosis +treatment

A

Generally neutralising autoantibody against FVIII

Diagnosed with inhibitor assays

Treatment:
Human FVIII not effective
Recombinant porcine FVIII better
Immunosuppressive therapy

Clinical Biomedicine (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions